Research Article
BibTex RIS Cite

Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi

Year 2019, Volume: 17 Issue: 2, 279 - 289, 02.08.2019

Abstract

GİRİŞ ve AMAÇ: Sistemik başlangıçlı juvenile
idiyopatik artrit (JİA) nadir görülen bir JİA alt tipi olarak kabul edilir ve
diğer JİA alt gruplarından farklı olarak otoinflamatuar hastalık grubunda
değerlendirilir. Ülkemizde sistemik başlangıçlı JİA ile ilgili az sayıda
çalışma bulunmaktadır. Bu çalışmada kliniğimizde sistemik başlangıçlı JİA
tanılı olguların klinik ve laboratuvar özelliklerinin araştırılması
amaçlanmıştır.

YÖNTEM ve GEREÇLER: Kliniğimizde takip edilen 303 JİA tanılı olgu tarandı ve 18
sistemik başlangıçlı JİA tanılı olgu (%5,9) tespit edildi. Olguların verileri
elektronik dosya kayıtlarından geriye dönük olarak taranarak elde edildi.

BULGULAR: Olguların yaşları ortanca 11,7 yıl (5,3-20 yıl) ve kız erkek oranı
4/14 idi. Tanı yaşları ortanca 4,9 yıl (1,5-15,2 yıl) ve tanıda gecikme süresi
ortanca 4 hafta (2 hafta-10,8 yıl) olarak saptandı. En sık başvuru yakınması
olguların tümünde görülen ateş ve artrit (n=18, %100) idi. Bir olguda
monoartrit görülürken diğer olgularda birden fazla eklem tutulumu vardı. En sık
görülen form monosiklik formdu (n=7, %38,8). Olgularda görülen en sık
komplikasyon boy kısalığı (n=4, %22,2) idi ve makrofaj aktivasyon sendromu 2
olguda (%11,1) gelişti. Tedavide en sık steroidler (n=17, %94,4) kullanılırken
biyolojik tedavi 7 olguda (%38,8) kullanıldı. Bu olguların son tedavilerine
bakıldığında; 5 olgunun tocilizumab, bir olgunun canakinumab aldığı, kalan 1
olgunun ise takibi bıraktığı saptandı. Takipten çıkan bir olgu dışında tüm
olgularda remisyon sağlandı.

TARTIŞMA ve SONUÇ: Sistemik başlangıçlı JİA, diğer JİA alt gruplarına kıyasla
daha az olarak görülmektedir. Hastalık enfeksiyon hastalıkları ve diğer
inflamatuar hastalıklarla karışabildiği için tanı gecikebilmektedir.
Kortikosteroid tedavisine çoğunlukla olumlu yanıt alınmakla birlikte steroide
dirençli olgularda anti IL-6 ve anti IL-1 ilaçlar etkili olabilmektedir

References

  • 1. Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet 2011;377:2138-49.
  • 2. Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet 2007; 369: 767-78.
  • 3. Thomson W, Barrett JH, Donn R, et al. British Paediatric Rheumatology Study Group. Juvenile idiopathic arthritis classified by the ILAR criteria: HLA associations in UK patients. Rheumatology (Oxford). 2002 Oct;41(10):1183-9.
  • 4. Masters SL, Simon A, Aksentijevich I, Kastner DL. Horror autoinfl ammaticus: the molecular pathophysiology of autoinfl ammatory disease (*). Annu Rev Immunol 2009; 27: 621-68.
  • 5. Giancane G, Consolaro A, Lanni S, Davì S, Schiappapietra B, Ravelli A. Juvenile Idiopathic Arthritis: Diagnosis and Treatment. Rheumatol Ther. 2016;3(2):187-207.
  • 6. Martini A. Systemic juvenile idiopathic arthritis. Autoimmun Rev 2012;12(1):56-9.
  • 7. Gurion R, Lehman TJ, Moorthy LN. Systemic arthritis in children: a review of clinical presentation and treatment. Int J Inflam 2011;2012:271569.
  • 8. Modesto C, Antón J, Rodriguez B, et al. Incidence and prevalence of juvenile idiopathic arthritis in Catalonia (Spain). Scand J Rheumatol 2010;39(6):472-9.
  • 9. Sawhney S, Magalhaes CS. Paediatric rheumatology – a global perspective. Best Pract Res Clin Rheumatol. 2006;20(2):201-21.
  • 10. Seth V, Kabra SK, Semwal OP, Jain Y. Clinico-immunological profile in juvenile rheumatoid arthritis--an Indian experience. Indian J Pediatr 1996;63(3):293-300.
  • 11. Fujikawa S, Okuni M. Clinical analysis of 570 cases with juvenile rheumatoid arthritis: results of a nationwide retrospective survey in Japan. Acta Paediatr Jpn 1997;39(2):245-9.
  • 12. Cakır N, Pamuk ÖN, Derviş E, et al. The prevalences of some rheumatic diseases in western Turkey: Havsa study. Rheumatol Int 2012;32(4):895-908.
  • 13. Şen V, Ece A, Uluca Ü, et al. Evaluation of children with juvenile idiopathic arthritis in southeastern Turkey: a single center experience. Hippokratia 2015;19(1):63-8.
  • 14. Erguven M, Kaya B, Hamzah OY, Tufan F. Evaluation of immune response to hepatitis A vaccination and vaccine safety in juvenile idiopathic arthritis. J Chin Med Assoc 2011;74(5):205-8.
  • 15. Shenoi S, Wallace CA. Diagnosis and Treatment of Systemic Juvenile Idiopathic Arthritis. J Pediatr 2016;177:19-26.
  • 16. Cimaz R. Systemic-onset juvenile idiopathic arthritis. Autoimmun Rev 2016;15(9):931-4.
  • 17. Behrens EM, Beukelman T, Gallo L,et al. Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). J Rheumatol 2008;35:343-8.
  • 18. Kimura Y, Weiss JE, Haroldson KL, et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2013;65:745-52.
  • 19. Savolainen HA, Isomaki HA. Decrease in the number of deaths from secondary amyloidosis in patients with juvenile rheumatoid arthritis. J Rheumatol 1993;20:1201-3.
  • 20. Immonen K, Savolainen HA, Hakala M. Why can we no longer find juvenile idiopathic arthritis-associated amyloidosis in childhood or in adolescence in Finland. Scand J Rheumatol 2007;36:402-3.
  • 21. Hashkes PJ, Wright BM, Lauer MS, et al. Mortality outcomes in pediatric rheumatology in the US. Arthritis Rheum 2010;62:599-608.
  • 22. Frosch M, AhlmannM, Vogl T, et al. Themyeloidrelated proteins 8 and 14 complex, a novel ligand of toll-like receptor 4, and interleukin-1 beta form a positive feedback mechanism in systemic-onset juvenile idiopathic arthritis. Arthritis Rheum 2009;60:883-91.
  • 23. Wakil SM, Monies DM, Abouelhoda M, et al. Association of a mutation in LACC1 with a monogenic form of systemic juvenile idiopathic arthritis. Arthritis Rheumatol 2015;67:288-95.
  • 24. Ombrello MJ, Remmers EF, Tachmazidou I, et al. HLADRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis. Proc Natl Acad Sci USA 2015;112:15970–5.
  • 25. Chae JJ, Wood G, Masters SL, et al. The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1beta production. Proc Natl Acad Sci USA. 2006;103:9982-7.
  • 26. Lachmann HJ, Sengül B, Yavuzşen TU, et al. Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford) 2006 Jun;45(6):746-50.
  • 27. Kalyoncu M, Acar BC, Cakar N, et al. Are carriers for MEFV mutations "healthy"? Clin Exp Rheumatol 2006;24:S120-2.
  • 28. Ayaz NA, Ozen S, Bilginer Y, et al. MEFV mutations in systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford) 2009;48(1):23-5.
  • 29. Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997;40(10):1879-85.
  • 30. Otten MH, Prince FH, ArmbrustW, et al. Factors associated with treatment response to etanercept in juvenile idiopathic arthritis. JAMA 2011;306:2340-7.
  • 31. Woo P, Southwood TR, Prieur AM, et al. Randomized, placebo-controlled, crossover trial of low-dose oral methotrexate in children with extended oligoarticular or systemic arthritis. Arthritis Rheum 2000;43:1849-57.
  • 32. Shenoi S, Wallace CA. Tumor necrosis factor inhibitors in the management of juvenile idiopathic arthritis: an evidence-based review. Paediatr Drugs 2010;12:367-77.
  • 33. Quartier P, Taupin P, Bourdeaut F, et al. Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type. Arthritis Rheum 2003;48:1093-101.
  • 34. Kimura Y, Pinho P,Walco G, et al. Etanercept treatment in patients with refractory systemic onset juvenile rheumatoid arthritis. J Rheumatol 2005;32:935-42.
  • 35. Russo RA, Katsicas MM. Clinical remission in patients with systemic juvenile idiopathic arthritis treated with anti-tumor necrosis factor agents.J Rheumatol 2009;36:1078-82.
  • 36. Ringold S, Weiss PF, Beukelman T, et al. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologicmedications. Arthritis Rheum 2013;65:2499-512.

Evaluation of patients with systemic onset juvenile idiopathic arthritis

Year 2019, Volume: 17 Issue: 2, 279 - 289, 02.08.2019

Abstract

INTRODUCTION:
Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory
disease classified as a subtype of JIA. There are few studies on sJIA in our
country. The aim of this study was to investigate the clinical and laboratory
features of patients with sJIA.

METHODS: Three hundred three cases with JIA followed up in our clinic were
screened, 18 of them (5.9%) diagnosed with sJIA wereincluded to our study. The
data was obtained from the electronic file records retrospectively.

RESULTS: The median age of the patients was 11.7 years (5.3-20 years), the
female/male ratio was 4/14, and the median diagnosis age was 4.9 years
(1.5-15.2 years). The median diagnosis delay was 4 weeks (2 weeks-10.8 years).
The most common complaints were fever and arthritis which were seen in all
cases (n = 18, 100%). One patient had monoarthritis while the other cases had
more than one joint involvement. The most common form was the monocyclic form
(n = 7, 38.8%). The most common complications were short stature and macrophage
activation syndrome which developed in 2 cases. Almost all patients were
treated by corticosteroids (n = 17, 94.4%), biological therapy was used in 7
patients (38.8%). Biological treatments were tocilizumab in five patients,
canakinumab in one and anakinra in 1 one patient. Remission was achieved in all
cases except one patient who was out of follow-up.

DISCUSSION AND CONCLUSION: SJIA can be challenging to diagnose. Fever and rash
in sJIA clinic may inadvertently be attributed to infectious disease.
Corticosteroids were the main treatment modalities in treatment, whereas in
resistant cases biologicals were highly effective and safe.

References

  • 1. Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet 2011;377:2138-49.
  • 2. Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet 2007; 369: 767-78.
  • 3. Thomson W, Barrett JH, Donn R, et al. British Paediatric Rheumatology Study Group. Juvenile idiopathic arthritis classified by the ILAR criteria: HLA associations in UK patients. Rheumatology (Oxford). 2002 Oct;41(10):1183-9.
  • 4. Masters SL, Simon A, Aksentijevich I, Kastner DL. Horror autoinfl ammaticus: the molecular pathophysiology of autoinfl ammatory disease (*). Annu Rev Immunol 2009; 27: 621-68.
  • 5. Giancane G, Consolaro A, Lanni S, Davì S, Schiappapietra B, Ravelli A. Juvenile Idiopathic Arthritis: Diagnosis and Treatment. Rheumatol Ther. 2016;3(2):187-207.
  • 6. Martini A. Systemic juvenile idiopathic arthritis. Autoimmun Rev 2012;12(1):56-9.
  • 7. Gurion R, Lehman TJ, Moorthy LN. Systemic arthritis in children: a review of clinical presentation and treatment. Int J Inflam 2011;2012:271569.
  • 8. Modesto C, Antón J, Rodriguez B, et al. Incidence and prevalence of juvenile idiopathic arthritis in Catalonia (Spain). Scand J Rheumatol 2010;39(6):472-9.
  • 9. Sawhney S, Magalhaes CS. Paediatric rheumatology – a global perspective. Best Pract Res Clin Rheumatol. 2006;20(2):201-21.
  • 10. Seth V, Kabra SK, Semwal OP, Jain Y. Clinico-immunological profile in juvenile rheumatoid arthritis--an Indian experience. Indian J Pediatr 1996;63(3):293-300.
  • 11. Fujikawa S, Okuni M. Clinical analysis of 570 cases with juvenile rheumatoid arthritis: results of a nationwide retrospective survey in Japan. Acta Paediatr Jpn 1997;39(2):245-9.
  • 12. Cakır N, Pamuk ÖN, Derviş E, et al. The prevalences of some rheumatic diseases in western Turkey: Havsa study. Rheumatol Int 2012;32(4):895-908.
  • 13. Şen V, Ece A, Uluca Ü, et al. Evaluation of children with juvenile idiopathic arthritis in southeastern Turkey: a single center experience. Hippokratia 2015;19(1):63-8.
  • 14. Erguven M, Kaya B, Hamzah OY, Tufan F. Evaluation of immune response to hepatitis A vaccination and vaccine safety in juvenile idiopathic arthritis. J Chin Med Assoc 2011;74(5):205-8.
  • 15. Shenoi S, Wallace CA. Diagnosis and Treatment of Systemic Juvenile Idiopathic Arthritis. J Pediatr 2016;177:19-26.
  • 16. Cimaz R. Systemic-onset juvenile idiopathic arthritis. Autoimmun Rev 2016;15(9):931-4.
  • 17. Behrens EM, Beukelman T, Gallo L,et al. Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). J Rheumatol 2008;35:343-8.
  • 18. Kimura Y, Weiss JE, Haroldson KL, et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2013;65:745-52.
  • 19. Savolainen HA, Isomaki HA. Decrease in the number of deaths from secondary amyloidosis in patients with juvenile rheumatoid arthritis. J Rheumatol 1993;20:1201-3.
  • 20. Immonen K, Savolainen HA, Hakala M. Why can we no longer find juvenile idiopathic arthritis-associated amyloidosis in childhood or in adolescence in Finland. Scand J Rheumatol 2007;36:402-3.
  • 21. Hashkes PJ, Wright BM, Lauer MS, et al. Mortality outcomes in pediatric rheumatology in the US. Arthritis Rheum 2010;62:599-608.
  • 22. Frosch M, AhlmannM, Vogl T, et al. Themyeloidrelated proteins 8 and 14 complex, a novel ligand of toll-like receptor 4, and interleukin-1 beta form a positive feedback mechanism in systemic-onset juvenile idiopathic arthritis. Arthritis Rheum 2009;60:883-91.
  • 23. Wakil SM, Monies DM, Abouelhoda M, et al. Association of a mutation in LACC1 with a monogenic form of systemic juvenile idiopathic arthritis. Arthritis Rheumatol 2015;67:288-95.
  • 24. Ombrello MJ, Remmers EF, Tachmazidou I, et al. HLADRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis. Proc Natl Acad Sci USA 2015;112:15970–5.
  • 25. Chae JJ, Wood G, Masters SL, et al. The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1beta production. Proc Natl Acad Sci USA. 2006;103:9982-7.
  • 26. Lachmann HJ, Sengül B, Yavuzşen TU, et al. Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford) 2006 Jun;45(6):746-50.
  • 27. Kalyoncu M, Acar BC, Cakar N, et al. Are carriers for MEFV mutations "healthy"? Clin Exp Rheumatol 2006;24:S120-2.
  • 28. Ayaz NA, Ozen S, Bilginer Y, et al. MEFV mutations in systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford) 2009;48(1):23-5.
  • 29. Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997;40(10):1879-85.
  • 30. Otten MH, Prince FH, ArmbrustW, et al. Factors associated with treatment response to etanercept in juvenile idiopathic arthritis. JAMA 2011;306:2340-7.
  • 31. Woo P, Southwood TR, Prieur AM, et al. Randomized, placebo-controlled, crossover trial of low-dose oral methotrexate in children with extended oligoarticular or systemic arthritis. Arthritis Rheum 2000;43:1849-57.
  • 32. Shenoi S, Wallace CA. Tumor necrosis factor inhibitors in the management of juvenile idiopathic arthritis: an evidence-based review. Paediatr Drugs 2010;12:367-77.
  • 33. Quartier P, Taupin P, Bourdeaut F, et al. Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type. Arthritis Rheum 2003;48:1093-101.
  • 34. Kimura Y, Pinho P,Walco G, et al. Etanercept treatment in patients with refractory systemic onset juvenile rheumatoid arthritis. J Rheumatol 2005;32:935-42.
  • 35. Russo RA, Katsicas MM. Clinical remission in patients with systemic juvenile idiopathic arthritis treated with anti-tumor necrosis factor agents.J Rheumatol 2009;36:1078-82.
  • 36. Ringold S, Weiss PF, Beukelman T, et al. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologicmedications. Arthritis Rheum 2013;65:2499-512.
There are 36 citations in total.

Details

Primary Language Turkish
Journal Section Araştırma
Authors

Şükrü Çekiç This is me

Yasin Karalı This is me

Sara Şebnem Kılıç

Publication Date August 2, 2019
Published in Issue Year 2019 Volume: 17 Issue: 2

Cite

APA Çekiç, Ş., Karalı, Y., & Kılıç, S. Ş. (2019). Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi. Güncel Pediatri, 17(2), 279-289. https://doi.org/10.32941/pediatri.600334
AMA Çekiç Ş, Karalı Y, Kılıç SŞ. Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi. Güncel Pediatri. August 2019;17(2):279-289. doi:10.32941/pediatri.600334
Chicago Çekiç, Şükrü, Yasin Karalı, and Sara Şebnem Kılıç. “Sistemik başlangıçlı Juvenil Idiyopatik Artrit tanılı olguların değerlendirilmesi”. Güncel Pediatri 17, no. 2 (August 2019): 279-89. https://doi.org/10.32941/pediatri.600334.
EndNote Çekiç Ş, Karalı Y, Kılıç SŞ (August 1, 2019) Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi. Güncel Pediatri 17 2 279–289.
IEEE Ş. Çekiç, Y. Karalı, and S. Ş. Kılıç, “Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi”, Güncel Pediatri, vol. 17, no. 2, pp. 279–289, 2019, doi: 10.32941/pediatri.600334.
ISNAD Çekiç, Şükrü et al. “Sistemik başlangıçlı Juvenil Idiyopatik Artrit tanılı olguların değerlendirilmesi”. Güncel Pediatri 17/2 (August 2019), 279-289. https://doi.org/10.32941/pediatri.600334.
JAMA Çekiç Ş, Karalı Y, Kılıç SŞ. Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi. Güncel Pediatri. 2019;17:279–289.
MLA Çekiç, Şükrü et al. “Sistemik başlangıçlı Juvenil Idiyopatik Artrit tanılı olguların değerlendirilmesi”. Güncel Pediatri, vol. 17, no. 2, 2019, pp. 279-8, doi:10.32941/pediatri.600334.
Vancouver Çekiç Ş, Karalı Y, Kılıç SŞ. Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi. Güncel Pediatri. 2019;17(2):279-8.